249 research outputs found

    Two sisters with familial dyskeratotic comedones

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    Familial dyskeratotic comedones is a rare autosomal dominant genodermatosis, which presents with disseminated keratotic papules and comedo-like lesions. The disease usually manifests in childhood. The histology of the lesions is characterized by a crater-like invagination of the epidermis filled with parakeratotic keratin. The epidermis usually shows acantholysis. So far the causative gene defect is unknown. We report on a new family with two affected sisters. The more severely affected woman was treated with oral retinoids without any effect. Afterwards some regions were successfully treated by CO2 laser-therapy

    Large altitudinal increase in tree root/shoot ratio in tropical mountain forests of Ecuador

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    Tropical rain forests decrease in tree height and aboveground biomass (AGB) with increasing elevation. The causes of this phenomenon remain insufficiently understood despite a number of explanations proposed including direct or indirect effects of low temperature on carbon acquisition and carbon investment, adverse soil conditions and impaired nutrient supply. For analysing altitudinal patterns of aboveground/belowground carbon partitioning, we measured fine (<2mm in diameter) and coarse root (2-5mm) biomass and necromass and leaf area index (LAI), and estimated AGB, from stand structural parameters in five tropical mountain rain forests at 1050, 1540, 1890, 2380 and 3060 m along an altitudinal transect in the South Ecuadorian Andes. Average tree height and AGB were reduced to less than 50% between 1050 and 3060 m, LAI decreased from 5.1 to 2.9. The leaf area reduction must have resulted in a lowered canopy carbon gain and thus may partly explain the reduced tree growth in the high-elevation stands. In contrast, both fine and coarse root biomass significantly increased with elevation across this transect. The ratio of root biomass (fine and coarse) to AGB increased more than ten-fold from 0.04 at 1050 m to 0.43 at 3060 m. Under the assumption that fine root biomass does reflect root productivity, our data indicate a marked belowground shift in C allocation with increasing elevation. Possible explanations for this allocation shift are discussed including reduced N supply due to low temperatures, water logging or adverse soil chemical. conditions. We conclude that the fine root system and its activity may hold the key for understanding the impressive reduction in tree size along tropical mountain slopes in Ecuador. Analyses of fine root turnover and longevity in relation to environmental factors along altitudinal transects in tropical mountains are urgently needed. (C) 2006 Gesellschaft fur Okologie. Published by Elsevier GnnbH. All rights reserved

    Efectividad del entrenamiento de fuerza en niños y adolescentes, un meta-análisis

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    Fil: García, Christian Martín. Universidad Nacional de La Plata. Facultad de Humanidades y Ciencias de la Educación; Argentina

    CD4+CD56+blastic tumor of the skin: cytogenetic observations and further evidence of an origin from plasmocytoid dendritic cells

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    We here report on a case of a blastic tumor, recently described to belong to a new entity sharing phenotypic similarities with blood derived plasmocytoid dendritic cells and formerly regarded as belonging to the group of natural killer cell lymphomas. Besides immunophenotypic characteristics such as the absence of T- cell markers and almost complete absence of markers of the myeloid lineage, these tumors express CD4, CD56 and CD123, the receptor for interleukin-3. Moreover, using the comparative genomic hybridisation technique, CGH, we demonstrate a gain of chromosome 7q, 22 and a loss of chromosome 3p and 13q. Since this type of hematologic disorder often shows its primary manifestation in the skin and often runs a rapidly fatal course, it is important to distinguish this from other types of primary cutaneous lymphomas

    Idiopathic calcinosis cutis with extended leg ulcers. Successful therapy with aluminium hydroxide

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    We report on a 51-year-old woman with an idiopathic calcinosis cutis. She developed longstanding multiple, extended and very painful ulcers on both lower legs. The ulcerations showed a bizarre configuration surrounded by a livedo racemosa vessel pattern and they were extremely resistant to any therapy. The characteristical histopathological features with occlusion of small and medium-sized blood vessels and massive calcification of the intima, ischemic necrosis and calcifications of subcutanous fat distant from blood vessels led to the diagnosis. Moreover, we detected multiple calcifications in the soft tissue radiologically. We did not find any underlying cause for the calcinosis cutis. Despite serum phosphate levels in the upper normal range therapy with oral aluminium hydroxide was started. There were no adverse effects from therapy. After 12 weeks the lesions showed continious improvement accompanied by a significant pain relief. The ulcers healed completely after covering with split thickness skin grafts, This case supports the efficiency of oral phosphate-binding agents in the therapy of calcinosis cutis

    Idiopathic calcinosis cutis with extended leg ulcers. Successful therapy with aluminium hydroxide

    No full text
    We report on a 51-year-old woman with an idiopathic calcinosis cutis. She developed longstanding multiple, extended and very painful ulcers on both lower legs. The ulcerations showed a bizarre configuration surrounded by a livedo racemosa vessel pattern and they were extremely resistant to any therapy. The characteristical histopathological features with occlusion of small and medium-sized blood vessels and massive calcification of the intima, ischemic necrosis and calcifications of subcutanous fat distant from blood vessels led to the diagnosis. Moreover, we detected multiple calcifications in the soft tissue radiologically. We did not find any underlying cause for the calcinosis cutis. Despite serum phosphate levels in the upper normal range therapy with oral aluminium hydroxide was started. There were no adverse effects from therapy. After 12 weeks the lesions showed continious improvement accompanied by a significant pain relief. The ulcers healed completely after covering with split thickness skin grafts, This case supports the efficiency of oral phosphate-binding agents in the therapy of calcinosis cutis

    Copper binding and conformation of the N-terminal octarepeats of the prion protein in the presence of DPC micelles as membrane mimetic

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    The prion protein is usually pictured as globular structured C-terminal domain that is linked to an extended flexible N-terminal tail. However, in its physiol. form, it is a glycoprotein tethered to the cell surface via a C-terminal GPI anchor. The low soly. of PrP even without GPI anchor and its strong tendency for aggregation has forced most structural investigations to be performed at low pH and mostly with N-terminally truncated variants. In the present study, we have used a synthetic peptide related to the PrP tetra-octarepeat region, i.e., the sequence (Pro-His-Gly-Gly-Gly-Trp-Gly-Gln)4, for NMR structural anal. of its preferred conformation in DPC micelles as membrane mimic. Well-defined and identical loops are obsd. between the four octarepeats that are linked by flexible Gly-Gly-Gly sequences. Interaction with the micelles is mainly through the tryptophan residues that appear to act as anchors. Copper binding to the peptide in the presence of DPC micelles revealed marked conformational rearrangements although binding to the micelles is preserved. Interestingly, titrn. expts. point to cooperative effects for the four binding sites. A destabilization of the DPC micelles by the peptide parallels the destabilizing effect of the prion protein on membranes so that the octarepeat region appears to be very membrane-active. How the physico-chem. properties reported here are linked to the function and significance of the prion protein remains a puzzle as long as the functional mechanism of the prion protein is not precisely elucidated. Nevertheless, our results emphasize the strong influence of the (membrane) environment on the PrP properties

    Narrative Strategies in Benedikte Naubert's Neue Volksmarchen der Deutschen

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    High frequency of primary cutaneous lymphomas associated with lymphoproliferative disorders of different lineage

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    In patients suffering from primary cutaneous lymphomas, secondary malignancies of various origin may develop. However, the frequency of a second neoplasm deriving from another lymphoid lineage is still unclear and may be underestimated. We screened all our patients with primary cutaneous lymphomas from a 4-year recruitment period for a coexisting secondary lymphoproliferative disorder. The cohort comprised of a total of 82 patients with primary cutaneous lymphomas, 62 with primary cutaneous T-cell lymphoma (CTCL), 18 with primary cutaneous B-cell lymphomas, and two with CD4+/CD56+ hematodermic neoplasm/blastic lymphomas. Seven patients (8.5%) were identified with a coexisting lymphoma of a different lymphoid lineage. Four patients with Sezary syndrome (SS) suffered from systemic B-cell lymphoma. Two of these developed SS after chemotherapy of their B-cell lymphoma. The other three patients with various types of skin lymphomas (SS, Mycosis fungoides [MF], primary cutaneous marginal zone lymphoma) developed Hodgkin's disease (hairy cell leukemia). Our data indicate that patients with primary cutaneous lymphomas have an elevated risk for the development of a secondary lymphoproliferative disorder even without previous chemotherapy. Possible explanations for this association include a genetic predisposition, alterations in early progenitor cells, underlying viral infections, and/or stimulation of a B-cell clone by the malignant helper T cells of the primary CTCL and vice versa
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